[Pulmonary lymphangiomyomatosis with tuberous sclerosis and endolymphatic stromal myosis of the uterus].

Pulmonary lymphangiomyomatosis is a rare disorder that affects only women of reproductive age. The lung shows numerous cystic changes and striking proliferation of smooth muscle around the airways, blood vessels, and lymphatic channels. We encountered a patient with pulmonary lymphangiomyomatosis who had repeated pneumothorax and bloody sputum over 10 years. The patient also had other lesions, including intracranial calcification, renal angiomyolipoma and endolymphatic stromal myosis of the uterus. The first two findings are known as tuberous sclerosis, and lymphangiomyomatosis is considered to be a form fruste of tuberous sclerosis. However, we know of no previous reports of uterine lesions in this disease. Lymphangiomyomatosis may be a systemic disorder of smooth muscle.